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首页> 外文期刊>British Journal of Haematology >The pathogenesis and management of the coagulopathy of acute promyelocytic leukaemia.
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The pathogenesis and management of the coagulopathy of acute promyelocytic leukaemia.

机译：急性早幼粒细胞白血病凝血病的发病机制和处理。

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摘要

Coagulopathy occurs in most patients with (APML) and is life-threatening; therefore prompt diagnosis and recognition of any coagulation defect is imperative. Unfortunately haemorrhage remains a major cause of early death, preventing some from reaching treatment. The coagulopathy is caused directly or indirectly by the leukaemic cells through expression of activators of coagulation and fibrinolysis, proteases and cytokine generation, compounded by failure of platelet production due to marrow invasion. At presentation the predominant feature is usually hyperfibrinolysis. Since the introduction of all-trans retinoic acid (ATRA), patient outcome has dramatically improved; yet, haemorrhagic complications remain the most frequent cause of mortality. Thrombotic complications occur but are less well recognized and potentially underreported. Supportive measures and prompt initiation of ATRA currently represent the mainstay of treatment of the coagulopathy in patients with suspected APML, but unanswered questions remain as to the optimal approach to further decrease the associated haemorrhagic and thrombotic risks. In particular, it is unclear how to best predict and monitor the coagulopathy; whether there is a role for the early use of antifibrinolytics; the most appropriate trigger for giving fibrinogen replacement and the value of low-dose anticoagulation to suppress coagulation activation once fibrinolysis has been suppressed.
机译：凝结病发生在大多数（APML）患者中，并且威胁生命。因此，必须迅速诊断和识别任何凝血缺陷。不幸的是，出血仍然是早期死亡的主要原因，阻止了某些人得到治疗。凝血病是由白血病细胞通过凝血和纤溶激活因子的表达，蛋白酶和细胞因子的产生直接或间接引起的，并由于骨髓侵袭导致血小板产生失败而加剧。在介绍时，主要特征通常是高纤蛋白溶解。自从引入全反式维甲酸（ATRA）以来，患者的预后得到了显着改善。然而，出血并发症仍然是最常见的死亡原因。发生血栓性并发症，但认识不到，并且可能被漏报。目前，支持性措施和迅速开始ATRA代表可疑APML患者的凝血病治疗的主要手段，但是关于进一步降低相关的出血和血栓形成风险的最佳方法仍未得到解答。特别是，尚不清楚如何最好地预测和监测凝血病。早期使用抗纤维蛋白溶解药是否有作用;纤维蛋白原被抑制后，最合适的触发因素是给予纤维蛋白原替代物和低剂量抗凝剂抑制凝血激活的价值。

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